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表现为快速进展性痴呆的多发性硬脑膜动静脉瘘

Multiple dural arteriovenous fistulas presenting as rapidly progressive dementia.

作者信息

Mendonça Nuno, Santos Gonçalo, Duro Diana, Machado Egídio, Goulão Augusto, Santana Isabel

机构信息

Department of Neurology, University Hospital of Coimbra, Coimbra, Portugal.

出版信息

Neurologist. 2012 May;18(3):130-2. doi: 10.1097/NRL.0b013e318251e695.

DOI:10.1097/NRL.0b013e318251e695
PMID:22549352
Abstract

INTRODUCTION

Dural arteriovenous fistulas (DAVFs) are important causes of neurological dysfunction and are many times misdiagnosed. Particularly in older populations, DAVFs may present with a selective cognitive dysfunction.

CASE REPORT

The authors describe a 70-year-old woman presenting with a rapidly progressive dementia, very similar in presentation to prion disease. Neuroimaging showed multifocal DAVFs associated with venous thrombosis and white matter changes, suggesting that impaired cerebral circulation due to venous hypertensive encephalopathy caused the patient's dementia. Prompt treatment of some of the abnormal shunts with endovascular embolization resulted in a clinically relevant improvement. Subsequent clinical improvement was achieved with anticoagulation, although no cause or predisposing factor was documented that could have led to the development of the venous thrombosis.

CONCLUSIONS

Neurologists should maintain a high degree of suspicion to avoid missing the diagnosis of DAVFs that are potentially treatable lesions.

摘要

引言

硬脑膜动静脉瘘(DAVFs)是神经功能障碍的重要原因,且多次被误诊。特别是在老年人群中,DAVFs可能表现为选择性认知功能障碍。

病例报告

作者描述了一名70岁女性,表现为快速进展性痴呆,临床表现与朊病毒病非常相似。神经影像学显示多灶性DAVFs伴有静脉血栓形成和白质改变,提示静脉性高血压脑病导致的脑循环受损引起了患者的痴呆。通过血管内栓塞对一些异常分流进行及时治疗,取得了临床上相关的改善。随后通过抗凝治疗实现了临床改善,尽管未记录到可能导致静脉血栓形成的病因或易感因素。

结论

神经科医生应保持高度怀疑,以避免漏诊可能可治疗的DAVFs病变。

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